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马凡综合征是一种较少见的遗传性结缔组织病.现将我们所遇的一家系9例报告如下.1 病例报告例1 先证者,男,28岁.身高178cm.1990年12月17日晚突发心前区及背部撕裂样剧痛.向两腰部放射入院.主动脉瓣听诊区闻舒张期叹气样杂音Ⅳ/6级,向心尖部传导.周围血管征阳性.二维超声心动图(UCG)示:主动脉根部内径(AOD)89mm,主动脉瓣(AV)关闭明显双线,心脏各腔室不大.胸部CT及主动脉造影等检查,确诊为Ⅰ型主动脉夹层动脉瘤且怀疑为马凡综合征.后因终末期全心衰竭并肾功能衰竭医治无效于1993年7月1日死亡.
Marfan syndrome is a rare hereditary connective tissue disease. Now we encounter a family of 9 cases are reported as follows .1 Case Report 1 proband, male, 28 years old. Height 178cm .1990 12 On the evening of the evening of January 17, there was an acute tear in the precordial area and the back, and the patient was admitted to both lumbar aortic valves.The aortic valve auscultation area sigh diastolic noise Ⅳ / 6, Dimensional echocardiography (UCG) showed aortic root diameter (AOD) of 89 mm, aortic valve (AV) closed significantly double line, the heart chamber is not large. Chest CT and aortic angiography, diagnosed as type I Arterial dissection aneurysms and suspected Marfan syndrome due to end-stage heart failure and renal failure treatment was invalid on July 1, 1993 death.